Rbc disorders 8
-
Upload
prasad-csbr -
Category
Health & Medicine
-
view
1.474 -
download
4
description
Transcript of Rbc disorders 8
Aplastic Anemia
Dr.CSBR.Prasad, M.D.,
Definition
• Aplastic anemia refers to a syndrome of chronic primary hematopoietic failure and attendant pancytopenia
– Anemia
– Neutropenia, and
– Thrombocytopenia
Mechanisms
Stem cell defect:
• Autoimmune (Most common)
• Inherited or
• Acquired
Major Causes of Aplastic Anemia
Aplastic Anemias
Fanconi anemia
• AR disorder
• Defect in DNA repair
• Marrow hypoplasia
• Hypoplasia of kidney, spleen
• Hypoplastic anomalies of bone – Thumb, Radius
Fanconi anemia
Aplastic Anemias - Pathogenesis
Environmental factors trigger the production of genetically altered stem cells
1. Immunologically mediated suppression of stem cells
By cytokines released by T cells
2. Intrinsic abnormality of stem cells
↓ proliferation & differentiating capacity
Pathophysiology of aplastic anemia
Aplastic Anemias - Morphology
• BM Aspiration – Dry tap
• BM biopsy – Diagnostic
• BM – markedly hypocellular
• Empty marrow spaces - fat, fibrous tissue, lymphocytes, plasma cells
Aplastic anemia
Morphology
Aplastic Anemias
PBS
• NCNC, slight macrocytosis
• Reticulocytosis is absent
• Leukopenia
• Thrombocytopenia
Clinical Features
• Can occur at any age
• Onset is usually insidious
• Initial manifestations vary depending on the affected cell line
• Splenomegaly is absent
• Reticulocytopenia is the rule
If it is present, the diagnosis of aplastic anemia should be
seriously questioned
Aplastic Anemia
• DD
– MDS
– Aleukemic leukemia
Treatment
• Allogenic BMT
• Antithymocyte globulin along with cyclosporine
Mary Curie
Pure Red Cell Aplasia
• It’s a primary marrow disorder in which only erythroid progenitors are suppressed
• Causes:
– Thymoma
– Large granular lymphocytic leukemia
– Drug exposures
– Autoimmune disorders
– Parvovirus infection B19
Myelophthisic anemia
Myelophthisic anemia
SOL destroys BM architecture & depresses its productive capacity
Causes
1. Metastatic cancer – lung, breast, prostate, thyroid
2. Multiple myeloma, leukemia, lymphoma
3. Myelofibrosis
PBS - Leukoerythroblastosis
Polycythemia
Polycythemia
• Myeloproliferative disorder
– Unregulated proliferation of erythroid elements in BM
– ↑ Erythrocyte concentration in peripheral blood
• Also ↑ other blood cells
• Synonyms: Polycythemia rubra vera, primary polycythemia, erythemia
Polycythemia
• Polycythemia Vera – primary
• Secondary Polycythemia – Appropriate to erythropoietin production
• High altitude
• COPD
• Obesity
– Inappropriate to erythropoietin production • Tumors – RCC, HCC, uterine leiomyoma
• Renal ischemia
– Familial Polycythemia
• Relative Polycythemia – Gaisbock’s syndrome
– Dehydration
Polycythemia Vera
• Unknown cause
• Possible mechanisms
– Erythropoietin independent proliferation of neoplastic stem cells
– Hypersensitivity of erythroid stem cells to erythropoietin
– Hypersensitivity of erythroid stem cells to insulin growth factors
– Abnormal GF that act on normal stem cells
Polycythemia Vera – C/F
• 40- 60 years
• M > F
• Gradual in onset
• Headache, weakness, pruritis, weight loss, fatigue
• GIT bleeding, menorrhagia, hemoptysis
• MI, Renal vein thrombosis, cerebral ischemia
Plethora of polycythemia
Polycythemia Vera – C/F
• Splenomegaly – 75%
• Hepatomegaly – 50%
• Hypertension – 50%
• Long standing cases – myelofibrosis – 30%
• 10% cases develop into acute leukemia
Polycythemia Vera – Lab findings
• Absolute Erythrocytosis: > 6-10millions/cumm
• Hemoglobin: > 18 gm%
• PCV > 48% in F, > 52% in M
• ESR – 2-3mm/ hour
• Reticulocyte count – normal / mildly increased
• Leukocytosis: > 12,000/mm3
• Platelet count - ↑
• PBS – NNBP, MCHC
Polycythemia Vera – Lab findings
• BM –
– hypercellular
– 95% cases - ↑ megakaryocytes
– ↑ reticulin
• Treatment
– Phlebotomy
– Myelosuppresive therapy
E N D
Dr.CSBR.Prasad, M.D.,
Associate Professor of Pathology,
Sri Devaraj Urs Medical College,
Kolar-563101,
Karnataka,
INDIA.